Kids with cystic fibrosis living longer

Children born and diagnosed with cystic fibrosis in 2010 can anticipate living longer than children with the condition born earlier, a new study reports. However maintaining the survival gains will be challenging, an accompanying editorial says.

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Researchers examined the Cystic Fibrosis Foundation Patient Registry, established in 1966, to study survival trends between 2000 and 2010 and estimated life expectancy for children born and diagnosed with cystic fibrosis in 2010. They found that the median age of patients in the registry increased from 14.3 years to 16.7 years during the decade and adjusted mortality decreased by 1.8% each year.

If mortality remains at 2010 levels, median survival for girls born and diagnosed in 2010 would be 37 years and 40 years for boys (males had a 19% lower adjusted mortality risk than females). If mortality continues to decline at the same rate as it did between 2000 and 2010, life expectancy would be more than 50 years.

The findings have “important implications for prognostic discussions,” the researchers note, and also for the healthcare system, which can anticipate growing numbers of adults with cystic fibrosis. An accompanying editorial, while applauding progress in care of cystic fibrosis patients, warns that ensuring the kind of care that has made longer survival possible “will not be simple or inexpensive.” The many complications associated with cystic fibrosis mean that caring for adults with the disease “requires a village,” the researchers write, and will entail substantial resources.

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